Everyone is affected differently by EB and everyone’s story is different. We hope the following stories about some members of the DEBRA family will help others gain an insight into the challenges those with EB encounter and overcome.
All White fan Saimon is over the moon New Zealand made it to the Football World Cup and was unbeaten. The Mount Albert 9 year-old’s favourite position is goalie and like millions of people around the world his favourite player is David Beckham.
There’s not much that Saimon doesn’t attempt and when he’s not playing football he’s out on his bike, scooter or skate board. He also loves playing games on his play station and the pc.
At school his favourite subjects are maths and spelling but it’s the opportunity to play football during the breaks that he enjoys the most.
Bay of Plenty is home to four generations of the Buchan and Hira whānau, five of whom, aged between 70 and six years old have a dominant Dystrophic strain of EB (DDEB).
Buchan and Hira whānau
The oldest family members have been part of the DEBRA whānau since its inception 30 years ago.
Like all forms of EB, Dominant Dystrophic affects everyone differently. In one of the branches of the family, the affects appear to be lessening with each new generation. For the Buchans and the Hiras DDEB is characterised by scaring on the extremities of the lower part of the body, however this can also extend to the nose, hands, elbows and eyes. With the onset of puberty the affects diminish however with advancing age it begins to affect the body more internally particularly in the throat, and some family members require dilatation operations to stretch the scaring in their throats.
Five year-old Hannah Riordan has learnt the hard way that she can’t climb and slide down poles. However her mum thinks it is doubtful the blisters caused by that escapade will be her last.
Hannah with her new DEBRA friend Maddison
Hannah’s EB is Recessive Dystrophic (RDEB). At times her skin appears perfectly fine and at other times it will break down, requiring bandaging to heal.
Louise Riordan describes her daughter as well rounded, “she loves fairies, dolls, her bike and cars. She isn’t physically deterred by anything.”
“I’m a bit worried as she is starting school this year and at primary school you’re left to fend for yourself more than you are at preschool. She is going to have to learn the limitations of her skin.”
To help with the transition to primary school the Riordans will take a copy of the book The Boy Who Dared by M.P.Garrett to help educate Hannah’s teachers and fellow students about EB.
Holly test-drives her dream car
15 year-old Holly lives in Christchurch with her mum, dad and older brother. She attends high school and dreams of becoming a textile designer – designing fabric patterns.
Holly goes to the gym once a week for physio. Like most girls her age Holly loves fashion, movies, her Nintendo DS, sewing, cellphones and mochas. But most of all, Holly loves her cat Tinkerbell.
23 year-old Ashley plans to take over the world of media. He’s starting in his home town of Waipu where he, with the help of nine others, runs Radio Waves on 99.1 FM. Not content with focusing on one form of media he has also worked part time for Channel North - the local regional television station.
Ashley on the air
Ashley’s dry sense of humour disarms everyone he meets and his ambitious motto ‘you’ve got to be in to win’ is infectious.
Always willing to push the boundaries, Ashley had his lower left leg amputated when it outwore its usefulness during his teenage years. Then in 2009 he became involved in a cell therapy trial which involved travelling to and from Sydney for the trials – Ashley will keep us updated when the results are released.
His latest hobby is target shooting with high powered air rifles.
Remember to tune your dial to 99.1 FM the next time you’re travelling through Waipu!
The Boy Who Dared by M.P. Garrett is an illustrated children’s book telling the story of a boy with EB.
Copies are available for sale from DEBRA NZ.